Mediastinal germ cell tumor

Jump to: navigation, search

WikiDoc Resources for Mediastinal germ cell tumor

Articles

Most recent articles on Mediastinal germ cell tumor

Most cited articles on Mediastinal germ cell tumor

Review articles on Mediastinal germ cell tumor

Articles on Mediastinal germ cell tumor in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Mediastinal germ cell tumor

Images of Mediastinal germ cell tumor

Photos of Mediastinal germ cell tumor

Podcasts & MP3s on Mediastinal germ cell tumor

Videos on Mediastinal germ cell tumor

Evidence Based Medicine

Cochrane Collaboration on Mediastinal germ cell tumor

Bandolier on Mediastinal germ cell tumor

TRIP on Mediastinal germ cell tumor

Clinical Trials

Ongoing Trials on Mediastinal germ cell tumor at Clinical Trials.gov

Trial results on Mediastinal germ cell tumor

Clinical Trials on Mediastinal germ cell tumor at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Mediastinal germ cell tumor

NICE Guidance on Mediastinal germ cell tumor

NHS PRODIGY Guidance

FDA on Mediastinal germ cell tumor

CDC on Mediastinal germ cell tumor

Books

Books on Mediastinal germ cell tumor

News

Mediastinal germ cell tumor in the news

Be alerted to news on Mediastinal germ cell tumor

News trends on Mediastinal germ cell tumor

Commentary

Blogs on Mediastinal germ cell tumor

Definitions

Definitions of Mediastinal germ cell tumor

Patient Resources / Community

Patient resources on Mediastinal germ cell tumor

Discussion groups on Mediastinal germ cell tumor

Patient Handouts on Mediastinal germ cell tumor

Directions to Hospitals Treating Mediastinal germ cell tumor

Risk calculators and risk factors for Mediastinal germ cell tumor

Healthcare Provider Resources

Symptoms of Mediastinal germ cell tumor

Causes & Risk Factors for Mediastinal germ cell tumor

Diagnostic studies for Mediastinal germ cell tumor

Treatment of Mediastinal germ cell tumor

Continuing Medical Education (CME)

CME Programs on Mediastinal germ cell tumor

International

Mediastinal germ cell tumor en Espanol

Mediastinal germ cell tumor en Francais

Business

Mediastinal germ cell tumor in the Marketplace

Patents on Mediastinal germ cell tumor

Experimental / Informatics

List of terms related to Mediastinal germ cell tumor

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: M-GCTs; Malignant germ cell tumors of the mediastinum;

Overview

Mediastinal germ cell tumor is a extragonadal tumor derived from germ cell remnants in the mediastinum and a cause of anterior mediastinal mass.[1][2] Malignant germ cell tumors of the mediastinum are uncommon, representing only 3 to 10% of tumors originating in the mediastinum. Mediastinal germ cell tumor arises from germ cells, which are normally involved in the formation of the gametes. The pathogenesis of mediastinal germ cell tumor is characterized by the abnormal migration of germ cells during embryogenesis. The most common cause of mediastinal germ cell tumor is teratoma. Other causes of mediastinal germ cell tumor, include: embryonal cell carcinoma, choriocarcinoma, yolk sac tumor, mixed germ cell tumor. Mediastinal germ cell tumor is more commonly observed among male patients between 15 to 30 years old. The most common risk factor in the development of mediastinal germ cell tumor is Klinefelter syndrome (47XXY). The majority of patients with mediastinal germ cell tumor are usually symptomatic at the time of diagnosis. Early clinical features include chest pain, dyspnea, and cough. The diagnosis of a mediastinal germ cell tumor should be considered in all young males with a mediastinal mass. Cisplatin-based chemotherapy is the medical treatment of choice for mediastinal germ cell tumors. Radical resection in conjunction with chemotherapy/radiation is the most common approach to the treatment of mediastinal germ cell tumor.

Historical Perspective

  • Mediastinal germ cell tumor was first described by Friedman in 1951.

Classification

  • Mediastinal germ cell tumor may be classified according to histopathological subtype in 2 groups:
  • Seminoma
  • Non-seminomatous germ cell tumours (NSGCT)
  • Embryonal cell carcinoma
  • Choriocarcinoma
  • Yolk sac tumor
  • Teratoma (most common)
  • Mixed germ cell tumor

Pathophysiology

  • The pathogenesis of mediastinal germ cell tumor is characterized by the abnormal migration of germ cells.
  • Mediastinal germ cell tumor arises from germ cells, which are normally involved in the formation of the gametes.
  • Genetic mutations associated with the development of mediastinal germ cell tumor, include:[3]
  • Sex chromosomal abnormalities
  • Loss of 1p, 4q, and 6q
  • Gain of 1q, 3, and 20q
  • On gross pathology, findings of mediastinal germ cell tumor may include:
  • Unencapsulated
  • Homogenous fleshy mass with indistinct boundaries
  • Large size (20 to 30 cm)
  • Invasion of adjacent structures
  • On microscopic histopathological analysis findings of mediastinal germ cell tumor, include:
  • Large tumor cells with clear cytoplasm
  • Prominent nucleoli

Causes

  • The most common cause of mediastinal germ cell tumor is teratoma.
  • Other causes of mediastinal germ cell tumor, include:
  • Embryonal cell carcinoma
  • Choriocarcinoma
  • Yolk sac tumour
  • Mixed germ cell tumour

Differentiating Mediastinal Germ Cell Tumor from Other Diseases

  • Mediastinal germ cell tumor must be differentiated from other diseases that cause chest pain, [clinical feature 2], and [clinical feature 3], such as:

Epidemiology and Demographics

  • Mediastinal germ cell tumor are uncommon
  • Mediastinal germ cell tumor represent only 3 to 10% of tumors originating in the mediastinum

Age

  • Mediastinal germ cell tumor is more commonly observed among young adults.
  • Mediastinal germ cell tumor is more commonly observed among patients between 15 to 30 years old.

Gender

  • Mediastinal germ cell tumor affects men and women equally.
  • Males are more commonly affected with mediastinal germ cell tumor than females.

Race

  • There is no racial predilection for mediastinal germ cell tumor.

Risk Factors

  • The most common risk factor in the development of mediastinal germ cell tumor is Klinefelter syndrome (47XXY).

Natural History, Complications and Prognosis

  • The majority of patients with malignant mediastinal germ cell tumor are usually symptomatic at the time of diagnosis
  • Early clinical features include chest pain, dyspnea, and cough.
  • If left untreated, the majority of patients with mediastinal germ cell tumor may progress to develop metastasis, malignant pleural effusion, and respiratory failure.
  • The most common complication of mediastinal germ cell tumor is superior vena cava syndrome.
  • Prognosis is generally poor, and the median survival rate of patients with mediastinal germ cell tumor is approximately 5 months.[4]

Diagnosis

Diagnostic Criteria

  • The diagnosis of mediastinal germ cell tumor is made with the following diagnostic criteria:
  • Hormone production (e.g. beta-HCG, AFP)
  • No evidence of a primary tumor in the testes or ovaries
  • Anterior mediastinal mass

Symptoms

  • Mediastinal germ cell tumors are usually asymptomatic and found incidentally.
  • Symptoms of mediastinal germ cell tumor may include the following:

Physical Examination

  • Patients with mediastinal germ cell tumor usually are well-appearing.
  • Physical examination may be remarkable for:[4]
  • Dull percussion
  • Tactile fremitus
  • Reduced chest expansion
  • Crackling or bubbling noises
  • Present whispered pectoriloquy

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of mediastinal germ cell tumor, include:[4]

Imaging Findings

  • Chest radiography is the initial imaging modality of choice for mediastinal germ cell tumor.
  • On chest radiography, findings of mediastinal germ cell tumor, include:[4]
  • Displaced anterior junction line
  • Obliterated cardiophrenic angles
  • Obtuse angles with the mediastinum
  • Obliterated retrosternal clear space
  • Effacement/ dense ascending aorta
  • On chest radiography, signs of mediastinal germ cell tumor, include:
  • Hilum Overlay Sign: hilar vessels through the mass (the mass does not arise from the hilum)
  • On CT, findings of mediastinal germ cell tumor, include:
  • Anterior mediastinal mass
  • Obtuse angles with the mediastinum
  • Enhancing septations

The image below demonstrates a case of mediastinal germ cell tumor


Other Diagnostic Studies

  • Mediastinal germ cell tumor may also be diagnosed using positron emission tomography.
  • Findings on positron emission tomography, include:
  • Useful to rule out the involvement of thyroid tissue.

Treatment

Medical Therapy

  • Cisplatin-based chemotherapy is the medical treatment of choice for mediastinal germ cell tumors.
  • Treatment selection depends on disease stage and size of mediastinal tumor.
  • Common therapies for mediastinal germ cell tumor, include:[5]
  • Mediastinal germ cell tumors are highly sensitive to radiation therapy and to combination chemotherapy.
  • Alternative treatment for mediastinal germ cell tumor, include:[5]
  • Primary radiotherapy in the absence of metastatic disease

Surgery

  • Surgery is the mainstay of therapy for mediastinal germ cell tumor.[5]
  • Small mediastinal germ cell tumor (usually asymptomatic) should undergo thoracotomy and attempted complete resection.
  • Radical resection in conjunction with chemotherapy/radiation is the most common approach to the treatment of mediastinal germ cell tumor.

Prevention

  • There are no primary preventive measures available for mediastinal germ cell tumor.
  • Once diagnosed and successfully treated, patients with mediastinal germ cell tumor are followed-up every year.
  • Follow-up testing for mediastinal germ cell tumor, includes chest radiography and tumoral biomarkers.

References

  1. Alan Sandler (1997). "Mediastinal Germ Cell Tumors". Semin Respir Crit Care Med. 18 (4): 383-392. doi:10.1055/s-2007-1009353. 
  2. "Mediastinal Germ Cell Tumor Imaging". 
  3. Schneider DT, Schuster AE, Fritsch MK, Calaminus G, Göbel U, Harms D, Lauer S, Olson T, Perlman EJ (2002). "Genetic analysis of mediastinal nonseminomatous germ cell tumors in children and adolescents". Genes Chromosomes Cancer. 34 (1): 115–25. PMID 11921289. 
  4. 4.0 4.1 4.2 4.3 Childs WJ, Goldstraw P, Nicholls JE, Dearnaley DP, Horwich A (1993). "Primary malignant mediastinal germ cell tumours: improved prognosis with platinum-based chemotherapy and surgery". Br. J. Cancer. 67 (5): 1098–101. PMC 1968447Freely accessible. PMID 8494705. 
  5. 5.0 5.1 5.2 Extragonadal Germ Cell Tumors Treatment–Health Professional Version (PDQ®) http://www.cancer.gov/types/extragonadal-germ-cell/hp/extragonadal-treatment-pdq Accessed on April 14, 2016

Linked-in.jpg